AB0647 Clinical phenotype in scleroderma patients with limited and diffuse cutaneous disease based on autoimmunity
Authors: C. Sieiro Santos, C. Moriano, I. González Fernández, X. E. Larco Rojas, C. Álvarez Castro, E. Diez Alvarez
Journal: Annals of the Rheumatic Diseases (2022)

Background Classically, anti-centromere antibodies (ACA) are associated with limited cutaneous involvement (lcSSc) and pulmonary hypertension, whereas anti-topoisomerase I (Sc70) are associated with diffuse skin involvement (dcSSc) and pulmonary fibrosis (ILD). Patients with lcSSc and Sc70 antibodies draw particular attention, which is why characterization of clinical phenotypes can help distinguish patient subgroups and assessing the prognosis of the disease. Objectives We aimed to characterize the clinical phenotype of patients with SSc based on autoantibodies. Methods We included patients with SSc, fulfilling the 2013 ACR/EULAR criteria, with disease duration ≤10 years. We have compared different subgroups of patients with lcSSc: Scl70-lcSSc (group1), ACA-lcSSc (group 2) and ANA-lcSSc (group 3), (Table 1). Next, we compared patients with Scl70-lcSSc (group 1) to Scl70-dcSSc (group 4) and ANA-lcSSC (group 3) to ANA-dcSSC (group 5). In the ANA subgroup we included patients with negative Scl70 and ACA antibodies. We have assessed the risk of ILD, myositis, scleroderma renal crisis, cardiac and gastrointestinal involvement, myositis, pulmonary hypertension (systolic pulmonary arterial pressure sPAP&gt;45 mmHg at transthoracic echocardiography or sPAP&gt;25 mmHg at right heart catheterization), cancer and all-cause-mortality. Table 1. Clinical characteristics of patients with lcSSc Scl70-lcSSc P value ACA-lcSSc P value ANA-lcSSc Female 9 (56.3%) 0.03 36 (83.7%) 0.80 12 (52%) Time from RP to SSc (years) 1.28 (0.25-3.5) 0.02 6.23 (3-9) 0.002 4.91 (2-5) Age at SSc onset 56.5 (48-66) 0.04 62.5 (54-73) 0.76 59.2 (44-66) CRP elevation 30 (22-39) 0.02 22 (16-29) 0.21 10 (6-19) mRSS 4 (2-4.2) 0.03 3.2 (2.1-4.0) 0.92 3.9 (2.5-4.2) Joint synovitis 6 (37.5%) 0.47 12 (28%) 0.71 10 (43%) Tendon friction rubs 2 (12.5%) 0.50 3 (7%) 0.19 0 Myositis 4 (25%) 0.04 2 (5%) 0.81 5 (21.7%) Gastrointestinal involvement 6 (37.5%) 0.19 27 (63%) 0.04 16 (69%) Renal crisis 4 (25%) 0.04 2 (5%) 0.18 2 (9%) ILD 10 (63%) 0.001 7 (16%) 0.04 7 (30%) Pulmonary hypertension 4 (25%) 0.47 7 (16%) 3 (13%) Arrythmia 5 (21.7%) 0.08 6 (14%) 0.35 7 (30%) Conduction defects 1 (6.3%) 0.59 5 (11.6%) 0.79 1 (4%) Diastolic disfunction 7 (43.8%) 0.36 17 (39.5%) 0.77 9 (39%) Immunosuppressants 10 (63%) 0.01 13 (30.2%) 0.52 12 (52%) Steroids 9 (56%) 0.04 12 (28%) 0.98 13 (56.6%) Cancer 8 (50%) 0.04 10 (23.2%) 0.22 7 (30%) Mortality 3 (18.8%) 0.04 6 (14%) 0.82 5 (21.7%) Results 103 SSc patients were included: 72 (69%) females, 82 (79%) lcSSc and 21 (20%) dcSSc. Among lcSSc patients, 43 (52%) had ACA, 16 (19%) Scl70 and 23 (28%) ANA. Among dcSSC patients, 9 (43%) had Scl70 and 12 (57%) had ANA. Scl70-lcSSc patients had significantly shorter time from Raynaud’s phenomenon (RP) to SSc diagnosis (p=0.02), younger age at SSc onset (p=0.04), higher mRSS (p=0.03), higher rate of myositis (p=0.04) and renal scleroderma crisis (p=0.04) than ACA-lcSSc patients. The risk of ILD in Scl70-lcSSC compared to ACA-lcSSC is 3.8 higher (95% IC 1.2-14.5) and 1.5 higher (95% IC 1.05-5.61) than ANA-lcSSC. All-cause mortality was higher in Scl70-lcSSC (p=0.04) compared to ACA-lcSSC. Scl70-dcSSC patients had a shorter time from RP to SSc diagnosis (p=0.02), higher CRP (p=0.04), mRSS (p=0.001), higher rate of myositis (p&lt;0.05) and ILD (p=0.04) and all-cause mortality (p=0.04) than Scl70-lcSSc patients, while renal and cardiac involvement was similar. ANA-dcSSC patients also had a shorter time from RP to SSc diagnosis (p=0.03), higher mRSS (p=0.02) and higher rate of ILD (p=0.02). Conclusion Scl70-lcSSc patients show the major organ involvement, followed by ANA-lcSSc and ACA-lcSSc. Scl70-dcSSc and ANA-dcSSC patients exhibit more cutaneous involvement and ILD than Scl70-lcSSc and ANA-lcSSc. These results may provide new ways to help in early diagnosis, management and assessing the prognosis of the disease. References [1]Zanatta E et al. POS0318 CLINICAL PHENOTYPE IN SCLERODERMA PATIENTS WITH ANTI-TOPOISOMERASE I POSITIVITY AND LIMITED CUTANEOUS FORM: DATA FROM THE EUSTAR DATABASE. Annals of the Rheumatic Diseases 2021;80:386-387. Disclosure of Interests None declared